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1

Inborn Errors of Metabolism with Hypoglycemia

Weinstein, David A., Steuerwald, Ulrike, De Souza, Carolina F.M., Derks, Terry G.J.

Journal:

Pediatric Clinics of North America

Year:

2018

Language:

english

File:

PDF, 540 KB

english, 2018

2

Evaluation of glycogen storage disease as a cause of ketotic hypoglycemia in children

Brown, Laurie M., Corrado, Michelle M., van der Ende, Rixt M., Derks, Terry G. J., Chen, Margaret A., Siegel, Sara, Hoyt, Kate, Correia, Catherine E., Lumpkin, Christopher, Flanagan, Theresa B., Carre

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 147 KB

english, 2015

3

Improved inflammatory bowel disease, wound healing and normal oxidative burst under treatment with empagliflozin in glycogen storage disease type Ib

GrÃ¼nert, Sarah C., Elling, Roland, Maag, BÃ¤rbel, Wortmann, Saskia B., Derks, Terry G. J., Hannibal, Luciana, Schumann, Anke, Rosenbaum-Fabian, Stefanie, Spiekerkoetter, Ute

Journal:

Orphanet Journal of Rare Diseases

Year:

2020

File:

PDF, 1009 KB

2020

4

A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers: from bedside to home site monitoring

Hoogeveen, Irene J., Peeks, Fabian, de Boer, Foekje, Lubout, Charlotte M. A., de Koning, Tom J., te Boekhorst, Sebastiaan, Zandvoort, Robert-Jan, Burghard, Rob, van Spronsen, Francjan J., Derks, Terry

Journal:

Journal of Inherited Metabolic Disease

Year:

2018

Language:

english

File:

PDF, 729 KB

english, 2018

5

Single amino acid supplementation in aminoacidopathies: a systematic review

van Vliet, Danique, Derks, Terry GJ, van Rijn, Margreet, de Groot, Martijn J, MacDonald, Anita, Heiner-Fokkema, M, van Spronsen, Francjan J

Journal:

Orphanet Journal of Rare Diseases

Year:

2014

Language:

english

File:

PDF, 306 KB

english, 2014

6

Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study

Schwahn, Bernd C, Van Spronsen, Francjan J, Belaidi, Abdel A, Bowhay, Stephen, Christodoulou, John, Derks, Terry G, Hennermann, Julia B, Jameson, Elisabeth, König, Kai, McGregor, Tracy L, Font-Montgom

Journal:

Year:

2015

Language:

english

File:

PDF, 1.06 MB

english, 2015

7

Inhibition of mitochondrial fatty acid oxidation in vivo only slightly suppresses gluconeogenesis but enhances clearance of glucose in mice

Terry G. J. Derks, Theo H. van Dijk, Aldo Grefhorst, Jan-Peter Rake, G. Peter A. Smit, Folkert Kuipers, Dirk-Jan Reijngoud

Journal:

Year:

2008

Language:

english

File:

PDF, 342 KB

english, 2008

8

Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl–CoA dehydrogenase (MCAD)–deficient mice

Hilde Herrema, Terry G. J. Derks, Theo H. van Dijk, Vincent W. Bloks, Albert Gerding, Rick Havinga, Uwe J. F. Tietge, Michael Müller, G. Peter A. Smit, Folkert Kuipers, Dirk-Jan Reijngoud

Journal:

Year:

2008

Language:

english

File:

PDF, 426 KB

english, 2008

9

MCAD deficiency; clinical and laboratory studies

Terry G. J. Derks

Journal:

Tijdschrift voor Kindergeneeskunde

Year:

2007

Language:

dutch

File:

PDF, 50 KB

dutch, 2007

10

Safe and unsafe duration of fasting for children with MCAD deficiency

Terry G. J. Derks, Francjan J. van Spronsen, Jan Peter Rake, Christian S. van der Hilst, Mark M. Span, G. Peter A. Smit

Journal:

European Journal of Pediatrics

Year:

2007

Language:

english

File:

PDF, 145 KB

english, 2007

11

The difference between observed and expected prevalence of MCAD deficiency in The Netherlands: a genetic epidemiological study

Derks, Terry G J, Duran, Marinus, Waterham, Hans R, Reijngoud, Dirk-Jan, ten Kate, Leo P, Smit, G Peter A

Journal:

European Journal of Human Genetics

Year:

2005

Language:

english

File:

PDF, 114 KB

english, 2005

12

Cost-Effectiveness of Neonatal Screening for Medium Chain acyl-CoA Dehydrogenase Deficiency: The Homogeneous Population of the Netherlands

Christian S. van der Hilst, Terry G.J. Derks, Dirk-Jan Reijngoud, G. Peter A. Smit, Elisabeth M. TenVergert

Journal:

The Journal of Pediatrics

Year:

2007

Language:

english

File:

PDF, 319 KB

english, 2007

13

The natural history of medium-chain acyl CoA dehydrogenase deficiency in the Netherlands: Clinical presentation and outcome

Terry G.J. Derks, Dirk-Jan Reijngoud, Hans R. Waterham, Willem-Jan M. Gerver, Maarten P. van den Berg, Pieter J.J. Sauer, G. Peter A. Smit

Journal:

The Journal of Pediatrics

Year:

2006

Language:

english

File:

PDF, 192 KB

english, 2006

14

MPV17: Fatal hepatocerebral presentation in a Brazilian infant

Célia Nogueira, Carolina F.M. de Souza, Antonette Husny, Terry G.J. Derks, Filippo M. Santorelli, Laura Vilarinho

Journal:

Molecular Genetics and Metabolism

Year:

2012

Language:

english

File:

PDF, 166 KB

english, 2012

15

LOCAL GOVERNMENT DEPARTMENT.

Derks, Terry G.J., Martens, Danielle H., Sentner, Christiaan P., van Rijn, Margreet, de Boer, Foekje, Smit, G. Peter A., van Spronsen, Francjan J.

Journal:

Year:

1898

Language:

english

File:

PDF, 215 KB

english, 1898

16

Dietary treatment of glycogen storage disease type Ia: Uncooked cornstarch and/or continuous nocturnal gastric drip-feeding?

Derks, Terry G.J., Martens, Danielle H., Sentner, Christiaan P., van Rijn, Margreet, de Boer, Foekje, Smit, G. Peter A., van Spronsen, Francjan J.

Journal:

Molecular Genetics and Metabolism

Year:

2013

Language:

english

File:

PDF, 112 KB

english, 2013

17

Recombinant phenylalanine ammonia lyase in phenylketonuria

van Spronsen, Francjan J, Derks, Terry G J

Journal:

Year:

2014

Language:

english

File:

PDF, 210 KB

english, 2014

18

Experimental evidence for protein oxidative damage and altered antioxidant defense in patients with medium-chain acyl-CoA dehydrogenase deficiency

Derks, Terry G. J., Touw, Catharina M. L., Ribas, Graziela S., Biancini, Giovana B., Vanzin, Camila S., Negretto, Giovanna, Mescka, Caroline P., Reijngoud, Dirk Jan, Smit, G. Peter A., Wajner, Moacir,

Journal:

Journal of Inherited Metabolic Disease

Year:

2014

Language:

english

File:

PDF, 235 KB

english, 2014

19

Synthesis and characterization of polynuclear chromium carbonyl tetraanions

McNeese, Timothy J., Mueller, Timothy E., Wierda, Derk A., Darensbourg, Donald J., Delord, Terry J.

Journal:

Inorganic Chemistry

Year:

1985

Language:

english

File:

PDF, 532 KB

english, 1985

20

Dietary management in glycogen storage disease type III: what is the evidence?

Derks, Terry G. J., Smit, G. Peter A.

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 209 KB

english, 2015

21

Mutations in RARS cause hypomyelination

Wolf, Nicole I., Salomons, Gajja S., Rodenburg, Richard J., Pouwels, Petra J. W., Schieving, Jolanda H., Derks, Terry G. J., Fock, Johanna M., Rump, Patrick, van Beek, Daphne M., van der Knaap, Marjo

Journal:

Annals of Neurology

Year:

2014

Language:

english

File:

PDF, 314 KB

english, 2014

22

BH4 treatment in BH4-responsive PKU patients: preliminary data on blood prolactin concentrations suggest increased cerebral dopamine concentrations

van Vliet, Danique, Anjema, Karen, Jahja, Rianne, de Groot, Martijn J., Liemburg, Geertje B., Heiner-Fokkema, M. Rebecca, van der Zee, Eddy A., Derks, Terry G.J., Kema, Ido P., van Spronsen, Francjan

Journal:

Molecular Genetics and Metabolism

Year:

2015

Language:

english

File:

PDF, 274 KB

english, 2015

23

In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases

Nalin, Tatiéle, Venema, Koen, Weinstein, David A., de Souza, Carolina F. M, Perry, Ingrid D. S., van Wandelen, Mario T. R., van Rijn, Margreet, Smit, G. Peter A., Schwartz, Ida V. D., Derks, Terry G.

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 490 KB

english, 2015

24

Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions

Derks, Terry G. J., van Rijn, Margreet

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 278 KB

english, 2015

25

Determination of amylose/amylopectin ratio of starches

Nalin, Tatiéle, Sperb-Ludwig, Fernanda, Venema, Koen, Derks, Terry G. J., Schwartz, Ida Vanessa D.

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 85 KB

english, 2015

26

Erratum to: In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases

Nalin, Tatiéle, Venema, Koen, Weinstein, David A., de Souza, Carolina F. M, Perry, Ingrid D. S., van Wandelen, Mario T. R., van Rijn, Margreet, Smit, G. Peter A., Schwartz, Ida V. D., Derks, Terry G.

Journal:

Journal of Inherited Metabolic Disease

Year:

2015

Language:

english

File:

PDF, 49 KB

english, 2015

27

Muscle Ultrasound in Patients with Glycogen Storage Disease Types I and III

Verbeek, Renate J., Sentner, Christiaan P., Smit, G. Peter A., Maurits, Natasha M., Derks, Terry G.J., van der Hoeven, Johannes H., Sival, Deborah A.

Journal:

Ultrasound in Medicine & Biology

Year:

2016

Language:

english

File:

PDF, 680 KB

english, 2016

28

Sweet and Sour Aspects of Medium-Chain Acyl CoA Dehydrogenase Deficiency

Derks, Terry G.J.

Journal:

Year:

2010

Language:

english

File:

PDF, 84 KB

english, 2010

29

Analysis of body composition and nutritional status in Brazilian phenylketonuria patients

Mazzola, Priscila Nicolao, Nalin, Tatiele, Castro, Kamila, van Rijn, Margreet, Derks, Terry G.J., Perry, Ingrid D.S., Mainieri, Alberto Scofano, Schwartz, Ida Vanessa D.

Journal:

Molecular Genetics and Metabolism Reports

Year:

2016

Language:

english

File:

PDF, 249 KB

english, 2016

30

Inborn Errors of Metabolism That Cause Sudden Infant Death: A Systematic Review with Implications for Population Neonatal Screening Programmes

van Rijt, Willemijn J., Koolhaas, Genevi�ve D., Bekhof, Jolita, Heiner Fokkema, M. Rebecca, de Koning, Tom J., Visser, Gepke, Schielen, Peter C.J.I., van Spronsen, Francjan J., Derks, Terry G.J.

Journal:

Year:

2016

Language:

english

File:

PDF, 93 KB

english, 2016

31

Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response

Mazzola, Priscila Nicolao, Teixeira, Bruno Costa, Schirmbeck, Gabriel Henrique, Reischak-Oliveira, Alvaro, Derks, Terry G.J., van Spronsen, Francjan J., Dutra-Filho, Carlos Severo, Schwartz, Ida Vanes

Journal:

Molecular Genetics and Metabolism Reports

Year:

2015

Language:

english

File:

PDF, 376 KB

english, 2015

32

Inflammatory Bowel Disease in Glycogen Storage Disease Type Ia

Lawrence, Nicole T., Chengsupanimit, Tayoot, Brown, Laurie M., Derks, Terry G.J., Smit, G. Peter A., Weinstein, David A.

Journal:

Journal of Pediatric Gastroenterology & Nutrition

Year:

2014

Language:

english

File:

PDF, 927 KB

english, 2014

33

Call for Nominations for INCOSE Fellows

Bahill, Terry, Kostek, Paul, Bol, Derk

Journal:

Year:

2002

Language:

english

File:

PDF, 106 KB

english, 2002

34

Neonates at risk of medium-chain acyl-CoA dehydrogenase deficiency: a perinatal protocol for use before population neonatal screening test results become available

van Rijt, Willemijn J., Jager, Emmalie A., van Spronsen, Francjan J., de Koning, Tom, Heiner-Fokkema, M. Rebecca, Derks, Terry G.J.

Journal:

Genetics in Medicine

Year:

2016

Language:

english

File:

PDF, 462 KB

english, 2016

35

Nine years of newborn screening for classical galactosemia in the Netherlands: Effectiveness of screening methods, and identification of patients with previously unreported phenotypes

Welling, Lindsey, Boelen, Anita, Derks, Terry G.J., Schielen, Peter C.J.I., de Vries, Maaike, Williams, Monique, Wijburg, Frits A., Bosch, Annet M.

Journal:

Molecular Genetics and Metabolism

Year:

2017

Language:

english

File:

PDF, 268 KB

english, 2017

36

Clinical and biochemical heterogeneity between patients with glycogen storage disease type IA: the added value of CUSUM for metabolic control

Peeks, Fabian, Steunenberg, Thomas A. H., de Boer, Foekje, Rubio-Gozalbo, M. Estela, Williams, Monique, Burghard, Rob, Rajas, Fabienne, Oosterveer, Maaike H., Weinstein, David A., Derks, Terry G. J.

Journal:

Journal of Inherited Metabolic Disease

Year:

2017

Language:

english

File:

PDF, 420 KB

english, 2017

37

Hepatocytes contribute to residual glucose production in a mouse model for glycogen storage disease type Ia

Hijmans, Brenda S., Boss, Andreas, van Dijk, Theo H., Soty, Maud, Wolters, Henk, Mutel, Elodie, Groen, Albert K., Derks, Terry G.J., Mithieux, Gilles, Heerschap, Arend, Reijngoud, Dirk-Jan, Rajas, Fab

Journal:

Year:

2017

Language:

english

File:

PDF, 1.49 MB

english, 2017

38

Glycogen Storage Diseases

Derks, Terry G. J., de Souza, Carolina Fischinger Moura

Journal:

Journal of Inborn Errors of Metabolism & Screening

Year:

2017

Language:

english

File:

PDF, 105 KB

english, 2017

39

Molybdenum cofactor deficiency type A: Prenatal monitoring using MRI

Lubout, Charlotte M.A., Derks, Terry G.J., Meiners, Linda, Erwich, Jan Jaap, Bergman, Klasina A., Lunsing, Roelineke J., Schwarz, Guenter, Veldman, Alex, van Spronsen, Francjan J.

Journal:

European Journal of Paediatric Neurology

Year:

2017

Language:

english

File:

PDF, 926 KB

english, 2017

40

Hepatic Glycogen Storage Diseases

Derks, Terry G. J., Nemeth, Antal, Adrian, Katrin, Arnell, Henrik, Roskjær, Ann Bech, Beijer, Eva, te Boekhorst, Sebastiaan, Heidenborg, Carina, Landgren, Marcus, Nilsson, Mikael, Papadopoulou, Domnik

Journal:

Journal of Inborn Errors of Metabolism & Screening

Year:

2017

Language:

english

File:

PDF, 132 KB

english, 2017

41

Glycogen storage disease type Ia: adult presentation with microcytic anemia and liver adenomas

Moest, Wouter, van der Deure, Wendy, Koster, Ted, Spee-Dropková, Marcela, Swart-Busscher, Linda, de Haas, Robbert J., Derks, Terry G.J.

Journal:

Year:

2018

Language:

english

File:

PDF, 1.43 MB

english, 2018

42

Proposal for an individualized dietary strategy in patients with very long-chain acyl-CoA dehydrogenase deficiency

Bleeker, Jeannette C., Kok, Irene L., Ferdinandusse, Sacha, de Vries, Maaike, Derks, Terry G. J., Mulder, Margot F., Williams, Monique, Rubio Gozalbo, Estela, Bosch, Annet M., van den Hurk, Dorine T.,

Journal:

Journal of Inherited Metabolic Disease

Year:

2018

Language:

english

File:

PDF, 854 KB

english, 2018

43

The need for additional care in patients with classical galactosaemia

Welling, Lindsey, Meester-Delver, Anke, Derks, Terry G., Janssen, Mirian C. H., Hollak, Carla E. M., de Vries, Maaike, Bosch, Annet M.

Journal:

Disability & Rehabilitation

Year:

2018

Language:

english

File:

PDF, 934 KB

english, 2018

44

Safety issues associated with dietary management in patients with hepatic glycogen storage disease

Steunenberg, Thomas A.H., Peeks, Fabian, Hoogeveen, Irene J., Mitchell, John J., Mundy, Helen, de Boer, Foekje, Lubout, Charlotte M.A., de Souza, Carolina F., Weinstein, David A., Derks, Terry G.J.

Journal:

Molecular Genetics and Metabolism

Year:

2018

Language:

english

File:

PDF, 358 KB

english, 2018

45

Pathogenic variants in glutamyl-tRNAGln amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder

Friederich, Marisa W., Timal, Sharita, Powell, Christopher A., Dallabona, Cristina, Kurolap, Alina, Palacios-Zambrano, Sara, Bratkovic, Drago, Derks, Terry G. J., Bick, David, Bouman, Katelijne, Chatf

Journal:

Nature Communications

Year:

2018

Language:

english

File:

PDF, 2.88 MB

english, 2018

46

Next-generation glycogen storage diseases

Derks, Terry G. J., Oosterveer, Maaike H., De Souza, Carolina F.

Journal:

Journal of Inherited Metabolic Disease

Year:

2018

Language:

english

File:

PDF, 310 KB

english, 2018

47

Proposal for an individualized dietary strategy in patients with very long-chain acyl-CoA dehydrogenase deficiency

Bleeker, Jeannette C., Kok, Irene L., Ferdinandusse, Sacha, de Vries, Maaike, Derks, Terry G.J., Mulder, Margot F., Williams, Monique, Gozalbo, Estela Rubio, Bosch, Annet M., van den Hurk, Dorine T.,

Journal:

Journal of Inherited Metabolic Disease

Year:

2019

Language:

english

File:

PDF, 1.27 MB

english, 2019

48

Impact of NBS for VLCAD deficiency on genetic, enzymatic and clinical outcomes

Bleeker, Jeannette C., Kok, Irene L., Ferdinandusse, Sacha, van der Pol, W. Ludo, Cuppen, Inge, Bosch, Annet M., Langeveld, Mirjam, Derks, Terry G. J., Williams, Monique, de Vries, Maaike, Mulder, Mar

Journal:

Journal of Inherited Metabolic Disease

Year:

2019

Language:

english

File:

PDF, 1.78 MB

english, 2019

49

Movement disorders and nonmotor neuropsychological symptoms in children and adults with classical galactosemia

Kuiper, Anouk, Grünewald, Stephanie, Murphy, Elaine, Coenen, Maraike A., Eggink, Hendriekje, Zutt, Rodi, Rubio-Gozalbo, Maria E., Bosch, Annet M., Williams, Monique, Derks, Terry G. J., Lachmann, Robi

Journal:

Journal of Inherited Metabolic Disease

Year:

2019

File:

PDF, 910 KB

2019

50

Movement disorders and nonmotor neuropsychological symptoms in children and adults with classical galactosemia

Kuiper, Anouk, Grünewald, Stephanie, Murphy, Elaine, Coenen, Maraike A., Eggink, Hendriekje, Zutt, Rodi, Rubio-Gozalbo, Maria E., Bosch, Annet M., Williams, Monique, Derks, Terry G. J., Lachmann, Robi

Journal:

Journal of Inherited Metabolic Disease

Year:

2019

Language:

english

File:

PDF, 910 KB

english, 2019